We performed a systematic review and meta-analysis of observational studies assessing quality of life in adolescents and young adults born with CHD compared with age-matched controls.

We carried out a systematic search of the literature published in Medline, Embase, PsychINFO, and the Cochrane Library’s Database (1990–2013); two authors independently extracted data from the included studies. We used the Newcastle–Ottawa scale for quality assessment of studies. A random effects meta-analysis model was used. Heterogeneity was assessed using the I2-test.

We included 18 studies with 1786 patients. The studies were of acceptable-to-good quality. The meta-analysis of six studies on quality of life showed no significant difference – mean difference: −1.31; 95% confidence intervals: −6.51 to +3.89, I2=90.9% – between adolescents and young adults with CHD and controls. Similar results were found in 10 studies not eligible for the meta-analysis. In subdomains, it seems that patients had reduced physical quality of life; however, social functioning was comparable or better compared with controls.

For the first time in a meta-analysis, we have shown that quality of life in adolescents and young adults with CHD is not reduced when compared with age-matched controls.

Cardiopulmonary bypass during tetralogy of Fallot corrective surgery is associated with oxidative stress, and contributes to peri-operative problems. Curcumin has been known as a potent scavenger of reactive oxygen species, which enhances the activity of antioxidants and suppresses phosphorylation of transcription factors involved in inflamation and apoptosis.

To evaluate the effects of curcumin as an antioxidant by evaluating the concentrations of malondialdehyde and glutathione, activity of nuclear factor-kappa B, c-Jun N-terminal kinase, caspase-3, and post-operative clinical outcomes.

Tetralogy of Fallot patients for corrective surgery were randomised to receive curcumin (45 mg/day) or placebo orally for 14 days before surgery. Malondialdehyde and glutathione concentrations were evaluated during the pre-ischaemia, ischaemia, re-perfusion phases, and 6 hours after aortic clamping-off. Nuclear factor-kappa B, c-Jun N-terminal kinase, and caspase-3, taken from the infundibulum, were assessed during the pre-ischaemia, ischaemia, and re-perfusion phases. Haemodynamic parameters were monitored until day 5 after surgery.

In all the observation phases, malondialdehyde and glutathione concentrations were similar between groups. There was no significant difference in nuclear factor-kappa B activity between the groups for three observations; however, in the curcumin group, c-Jun N-terminal kinase significantly decreased from the pre-ischaemia to the re-perfusion phases, and caspase-3 expression was lower in the ischaemia phase. Patients in the curcumin group had lower temperature and better ventricular functions, but no significant differences were found in mechanical ventilation day or length of hospital stay in the two groups.

Cardioprotective effects of curcumin may include inhibition of the c-Jun N-terminal kinase pathway and caspase-3 in cardiomyocytes, particularly in the ischaemia phase.

Our aim was to determine the optimal cut-off values, sensitivity, specificity, and diagnostic power of 12 echocardiographic parameters on the second day of life to predict subsequent ductal patency.

We evaluated preterm infants, born at ⩽32 weeks of gestation, starting on their second day of life, and they were evaluated every other day until ductal closure or until there were clinical signs of re-opening. We measured transductal diameter; pulmonary arterial diastolic flow; retrograde aortic diastolic flow; pulsatility index of the left pulmonary artery and descending aorta; left atrium and ventricle/aortic root ratio; left ventricular output; left ventricular flow velocity time integral; mitral early/late diastolic flow; and superior caval vein diameter and flow as well as performed receiver operating curve analysis.

Transductal diameter (>1.5 mm); pulmonary arterial diastolic flow (>25.6 cm/second); presence of retrograde aortic diastolic flow; ductal diameter by body weight (>1.07 mm/kg); left pulmonary arterial pulsatility index (⩽0.71); and left ventricle to aortic root ratio (>2.2) displayed high sensitivity and specificity (p<0.0001; area under the curve>0.9). Parameters with moderate sensitivity and specificity were as follows: left atrial to aortic root ratio; left ventricular output; left ventricular flow velocity time integral; and mitral early/late diastolic flow ratio (p<0.05; area under the curve 0.7–0.88). Descending aortic pulsatility index and superior caval vein diameter and flow (p>0.05) had low diagnostic value.

Left pulmonary arterial pulsatility index, left ventricle/aortic root ratio, and ductal diameter by body weight are useful adjuncts offering a broader outlook for predicting ductal patency.

Malignant arrhythmias are a major cause of sudden cardiac death in adults with congenital heart disease. We developed a model to serially investigate electrophysiological properties in an animal model of right ventricular hypertrophy and failure.

We created models of compensated (cHF; n=11) and decompensated (dHF; n=11) right ventricular failure in Wistar rats by pulmonary trunk banding. Healthy controls underwent sham operation (Control; n=13). Surface electrocardiography was recorded from extremities, and inducibility of ventricular tachycardia was evaluated in vivo by programmed stimulation. Isolated right ventricular myocardium was analysed for mRNA expression of selected genes.

Banding caused an increased mRNA expression of both connexin 43 and the voltage-gated sodium channel 1.5, as well as a prolongation of PQ, QRS and QTc intervals. Ventricular tachycardia was induced in the majority of banded animals compared with none in the healthy control group. No differences were found between the two degrees of failure in neither the electrophysiological parameters nor inducibility.

The electrophysiological properties of rat hearts subjected to pulmonary trunk banding were significantly changed with increased susceptibility to ventricular tachycardia, but no differences were found between compensated and decompensated right ventricular failure. Furthermore, we demonstrate that in vivo electrophysiological evaluation is a sensitive method to characterise the cardiac electric phenotype in an experimental rat model.

It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status – medical history and present medical status – on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents.

We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years.

Functional health status was assessed as medical history – that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures – and present medical status – assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form.

In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains.

Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.

The aim of this study was to investigate the potential role of N-terminal pro-brain natriuretic peptide in the assessment of shunt severity and invasive haemodynamic parameters in children with atrial septal defects and ventricular septal defects.

This is a prospective, controlled (n:62), observational study. Correlation analysis was performed between N-terminal pro-brain natriuretic peptide levels and various invasive haemodynamic measurements in 127 children (ventricular septal defect: 64; atrial septal defect: 63). A ratio of pulmonary to systemic blood flow (Qp/Qs⩾1.5) was considered to indicate a significant shunt.

Statistically significant relationship was found between the mean N-terminal pro-brain natriuretic peptide values of the patients, with Qp/Qs⩾1.5 in both defect types and control group. For ventricular septal defect, N-terminal pro-brain natriuretic peptide level⩾113.5 pg/ml was associated with high specificity and sensitivity for determining the significant shunt. In addition, the cut-off point for determining the significant shunt for atrial septal defect was 57.9 pg/ml. Significant positive correlation was found between all invasive haemodynamic parameters and N-terminal pro-brain natriuretic peptide levels in patients with ventricular septal defects. Whereas significant positive correlation was found only between mean pulmonary artery pressure, right ventricular end-diastolic pressure, and systemic pressure to pulmonary pressure ratio and N-terminal pro-brain natriuretic peptide levels in patients with atrial septal defects.

Our study demonstrated that the N-terminal pro-brain natriuretic peptide measurements could be used as a supporting parameter in determining significance of the shunt.

CHDs form a complex and heterogeneous group of clinical entities, with high morbidity and mortality. With the advancement of surgical and invasive techniques and clinical treatment, the survival of these patients has increased significantly, and there are reports of a high prevalence of ocular abnormalities in this group. The objective of this study was to estimate the prevalence of ocular findings in children and adolescents diagnosed with CHD.

A systematic search was conducted in the following databases: MEDLINE (via PubMed), EMBASE, and Cochrane CENTRAL, in addition to a manual search on studies published on the patient, from inception until August, 2014. Observational studies assessing the prevalence of ocular abnormalities in children and adolescents with CHDs were included.

Of the 2413 articles identified, eight were included, comprising a total of 1061 patients. Among them, the lowest and highest prevalences observed were 6.3 and 65%, respectively. The weighted average prevalence of ocular abnormalities was 32.5% (CI95% 19.3–49.3). Strabismus, cataracts, and retinopathy were the most frequently observed alterations.

The prevalence of ocular abnormalities in children and adolescents with CHDs was 32.5%, demonstrating that ocular consequences are not uncommon in this population and may have relevant clinical impact. These results reinforce the need for ophthalmological evaluation of patients with CHDs.

Debilitating patient-related non-cardiac co-morbidity cumulatively increases risk for congenital heart surgery. At our emerging programme, flexible surgical strategies were used in high-risk neonates and infants generally considered in-operable, in an attempt to make them surgical candidates and achieve excellent outcomes.

Between April, 2010 and November, 2013, all referred neonates (142) and infants (300) (average scores: RACHS 2.8 and STAT 3.0) underwent 442 primary cardiac operations: patients with bi-ventricular lesions underwent standard (n=294) or alternative (n=19) repair/staging strategies, such as pulmonary artery banding(s), ductal stenting, right outflow patching, etc. Patients with uni-ventricular hearts followed standard (n=96) or alternative hybrid (n=34) staging. The impact of major pre-operative risk factors (37%), standard or alternative surgical strategy, prematurity (50%), gestational age, low birth weight, genetic syndromes (23%), and major non-cardiac co-morbidity requiring same admission surgery (27%) was analysed on the need for extracorporeal membrane oxygenation, mortality, length of intubation, as well as ICU and hospital length of stays.

The need for extracorporeal membrane oxygenation (8%) and hospital survival (94%) varied significantly between surgical strategy groups (p=0.0083 and 0.028, respectively). In high-risk patients, alternative bi- and uni-ventricular strategies minimised mortality, but were associated with prolonged intubation and ICU stay. Major pre-operative risk factors and lower weight at surgery significantly correlated with prolonged intubation, hospital length of stay, and mortality.

In our emerging programme, flexible surgical strategies were offered to 53/442 high-risk neonates and infants with complex CHDs and significant non-cardiac co-morbidity, in order to buffer risk and achieve patient survival, although at the cost of increased resource utilisation.

Serum digoxin levels have limited utility for determining digoxin toxicity in adults. Paediatric data assessing the utility of monitoring serum digoxin concentration are scarce. We sought to determine whether serum digoxin concentrations are associated with signs and symptoms of digoxin toxicity in children.

We carried out a retrospective review of patients <19 years of age who received digoxin and had serum digoxin concentrations assessed between January, 2007 and June, 2013. Data collection included patient demographics, digoxin indication, serum digoxin concentrations, signs and symptoms of digoxin toxicity, electrocardiograms, and co-morbidities. Reviewers performing chart review and electrocardiogram analysis were blinded to digoxin levels. Descriptive statistical methods were used and comparisons were made between patients with and without toxic serum digoxin concentrations (>2 ng/ml).

There were 87 patients who met study criteria (male 46%, mean age 8.4 years). CHD was present in 67.8% and electrocardiograms were performed in 72.4% of the patients. The most common indication for digoxin toxicity was heart failure symptoms (61.5%). Toxic serum digoxin concentrations were present in 6.9% of patients (mean 2.6 ng/ml). Symptoms associated with digoxin toxicity occurred in 48.4%, with nausea/vomiting as the most common symptom (36.4%), followed by tachycardia (29.5%). Compared with those without toxic serum digoxin concentrations, significantly more patients with toxic serum digoxin concentrations were female (p=0.02). The presence of electrocardiogram abnormalities and/or signs and symptoms of digoxin toxicity was not significantly different between patients with and without serum digoxin concentrations (p>0.05).

Serum digoxin concentrations in children are not strongly associated with signs and symptoms of digoxin toxicity.

We sought to benchmark the utilisation of echocardiography in the outpatient evaluation of heart murmurs by evaluating two large paediatric cardiology centres.

Although criteria exist for appropriate use of echocardiography, there are no benchmarking data demonstrating its utilisation.

We performed a retrospective cohort study of outpatients aged between 0 and 18 years at the Sibley Heart Center Cardiology and the Children’s Hospital of Philadelphia Division of Cardiology, given a sole diagnosis of “innocent murmur” from 1 July, 2007 to 31 October, 2010. Using internal claims data, we compared the utilisation of echocardiography according to centre, patient age, and physician years of service.

Of 23,114 eligible patients (Sibley Heart Center Cardiology: 12,815, Children’s Hospital of Philadelphia Division of Cardiology: 10,299), 43.1% (Sibley Heart Center Cardiology: 45.2%, Children’s Hospital of Philadelphia Division of Cardiology: 40.4%; p<0.001) underwent echocardiography. There was wide variability in the utilisation of echocardiography by individual physicians at both centres (Sibley Heart Center Cardiology 18.3–85.5%, median 45.4%; Children’s Hospital of Philadelphia Division of Cardiology 13.9–81.8%, median 34.7%, p=0.45). Children in their first month of life represented the group with highest utilisation of echocardiography (62.3%), whereas children aged >1–5 years had the lowest utilisation (32.7%).

In two large paediatric cardiology practices, the overall utilisation of echocardiography by physicians with a sole diagnosis of innocent murmur was similar. There was significant and similar variability in utilisation by provider at both centres. Although these data serve as initial benchmarking, the variability in utilisation highlights the importance of appropriate use criteria.

Cardiopulmonary bypass is associated with systemic inflammatory response. Steroids suppress this response, although the therapeutic evidence remains controversial. We hypothesised that intravenous steroids in children undergoing open-heart surgery would decrease inflammation leading to better early post-operative outcomes. We conducted a randomised controlled trial to evaluate the trends in the levels of immunomodulators and their effects on clinical parameters.

To assess the effects of intravenous steroids on early post-operative inflammatory markers and clinical parameters in children undergoing open-heart surgery.

A randomised controlled trial involving 152 patients, from one month up to 18 years of age, who underwent open-heart surgery for congenital heart disease from April 2010–2012 was carried out. Patients were randomised and administered either three scheduled intravenous pulse doses of dexamethasone (1 mg/kg) or placebo. Blood samples were drawn at four time intervals and serum levels of inflammatory cytokines – Interleukin-6, 8, 10, 18, and tumour necrosis factor-alpha – were measured. Clinical parameters were also assessed.

Blood cytokine levels were compared between the dexamethasone (n=65) and placebo (n=64) groups. Interleukin-6 levels were lower at 6 and 24 hours post-operatively (p<0.001), and Interleukin-10 levels were higher 6 hours post-operatively (p<0.001) in the steroid group. Interleukin-8, 18, and tumour necrosis factor-alpha levels did not differ between the groups at any time intervals. The clinical parameters were similar in both the groups.

Dexamethasone caused quantitative suppression of Interleukin-6 and increased Interleukin-10 activation, contributing to reduced immunopathology, but it did not translate into clinical benefit in the short term.

The standard surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is the Rastelli operation. Recently, en bloc rotation of the arterial trunk, by cutting out the aortic and the pulmonary root in one block and by rotating it 180°, has been introduced as a new option for anatomical repair.

To evaluate the effects of this surgical method on the conduction system, pre-operative, post-operative, and follow-up electrocardiograms as well as patient charts were reviewed retrospectively. A total of 16 consecutive patients with transposition of the great arteries and left outflow tract obstruction were treated with en bloc rotation.

During the post-operative period, there were two patients with complete atrio-ventricular block, one with junctional ectopic tachycardia, one with ventricular tachycardia, and one with supraventricular tachycardia. None of the patients had a typical right bundle branch block pattern before surgery; however, this pattern was detectable after surgery in eight out of 16 patients (50%), which persisted during the follow-up. All patients without typical right bundle branch block pattern showed a median QRS duration of 65 ms (54–112 ms) before surgery, 62 ms (54–122 ms) after surgery, and 84 ms (66–128 ms) at the last follow-up visit. This compares well with a similar Rastelli cohort, where a right bundle branch block prevalence of 77% was reported. Out of 16 patients, 12 showed non-specific ST changes and negative T-waves, which persisted during follow-up with an unknown significance for the future.

Our data suggest that en bloc rotation of the arterial trunk seems not to have more negative effects on the conduction system than the Rastelli operation.

Many extremely low birth weight infants develop pulmonary hypertension late in their clinical course, and over 60% go undetected by early screening echocardiography. At present, no standardised screening protocol exists for detecting late pulmonary hypertension in extremely low birth weight infants. We assessed the utility of oxygen supplementation as a predictor of late pulmonary hypertension.

A retrospective single-centre review of extremely low birth weight infants with no evidence of CHD and those surviving for >30 days was performed. The association between oxygen ⩾30% at day of life 30 and diagnosis of late pulmonary hypertension was estimated with an odds ratio and 95% confidence interval using logistic regression. Doppler echocardiography was used to diagnose pulmonary hypertension in the infants.

A total of 230 infants met the study criteria. The incidence of late pulmonary hypertension was 8.3% (19/230). Infants with late pulmonary hypertension were more likely to have a lower mean birth weight (667.1±144 versus 799.3±140 g, p=0.001) and more likely to be small for gestational age (47.4 versus 14.2%, p=0.004). Oxygen requirement ⩾30% at day of life 30 was associated with increased risk of late pulmonary hypertension (odds ratio=3.77, 95% confidence interval=1.42–10.00, p=0.008) in univariate analysis and after adjusting for birth weight (odds ratio=2.47, 95% confidence interval=0.89–6.84, p=0.08).

The need of oxygen supplementation ⩾30% at day of life 30 may be a good screening tool for detecting late pulmonary hypertension in extremely low birth weight infants.

Following the Safe and Sustainable review of Paediatric Services in 2012/2013, National Health Service England recommended that local paediatric cardiology services should be provided by specially trained paediatricians with expertise in cardiology in all non-specialist hospitals.

To understand the variation in local paediatric cardiology services provided across district general hospitals in the United Kingdom.

An internet-based questionnaire was sent out via the Paediatrician with Expertise in Cardiology Special Interest Group and the Neonatologists with Interest in Cardiology and Haemodynamics contact databases and the National Health Service directory. Non-responders were followed-up via telephone.

The response rate was 80% (141 of 177 hospitals), and paediatricians with expertise in cardiology were available in 68% of those. Local cardiology clinics led by paediatricians with expertise in cardiology were provided in 96 hospitals (68%), whereas specialist outreach clinics were held in 123 centres (87%). A total of 11 hospitals provided neither specialist outreach clinics nor any local cardiology clinics led by paediatricians with expertise in cardiology. Paediatric echocardiography services were provided in 83% of the hospitals, 12-lead electrocardiogram in 96%, Holter electrocardiogram in 91%, and exercise testing in only 47% of the responding hospitals. Telemedicine facilities were established in only 52% of the centres, where sharing echocardiogram images via picture archiving and communication system was used most commonly.

There has been a substantial increase in the availability of paediatricians with expertise in cardiology since 2008. Most of the hospitals are well-supported by specialist cardiology centres via outreach clinics; however, there remains significant variation in the local paediatric cardiology services provided across district general hospitals in the United Kingdom.

Digital tonometry is designed to non-invasively screen for endothelial dysfunction by the detection of impaired flow-induced reactive hyperaemia in the fingertip. We determined whether digital reactive hyperaemia correlated with risk factors for atherosclerosis in two groups of children at increased risk for endothelial dysfunction.

A total of 15 obese children and 23 non-obese, dyslipidaemic children, 8–21 years of age, were enrolled, and their medical histories, anthropometric measurements, carotid wall thickness by means of ultrasonography, and fasting blood samples for cardiovascular risk factors were obtained. The standard endoPAT index of digital reactive hyperaemia was modified to reflect the true peak response or the integrated response of the entire post-occlusion period. In each group, age, sex, pubertal status, carotid wall thickness, and multiple cardiovascular risk factors were tested as predictors of endothelial dysfunction.

In the non-obese, dyslipidaemic group, but not in the obese group, both indices strongly correlated with height (r=0.55, p=0.007, by peak response) followed by weight, waist circumference, and age. In both groups, neither index of reactive hyperaemia significantly correlated with any other cardiovascular risk factor.

Contrary to the known age-related increase in atherosclerosis, digital reactive hyperaemia increased with age and its correlates in non-obese, dyslipidaemic children and was not related to other cardiovascular risk factors in either group. The reason for the lack of this relationship with age in obese children is unknown. The age-dependent physiology of digital microvascular reactivity and the endothelium-independent factors controlling the peak hyperaemic response need further study in children with a wide age range.

Dilatation of the ascending aorta is described in Turner’s syndrome with variable prevalence (6.8–32%). Reported series typically include patients with associated cardiac anomalies.

To characterise the prevalence, age of onset, and the progress of dilatation of the ascending aorta in Turner’s syndrome patients free of structural cardiac anomalies. Potential risk factors such as karyotype and growth hormone therapy were analysed for correlation with aortic dilatation.

We carried out a retrospective study with data collected from medical records and echocardiography studies. Patients with Tuner’s syndrome followed-up between 1992 and 2010 with at least two echocardiography studies were eligible. Patients with previous cardiac surgery or under anti-hypertensive medication were excluded. Ascending aorta diameter measurements were adjusted for body surface area, and dilatation was defined as Z-score>2.

The study population consisted of 44 patients, aged 11.9±7.4 years at the first echocardiogram and 17.9±7.3 years at the last follow-up, with a follow-up duration of 6.0±3.7 years. A total of 13 (29.5%) patients exhibited aortic dilatation during follow-up, suggesting an actuarial estimate of the freedom from aortic dilatation dropping from 86 to 70% and then to 37% at 10, 20, and 30 years of age, respectively. There was no statistically significant impact of karyotype or growth hormone therapy on aortic Z-score progression.

The prevalence of dilatation of the ascending aorta in Turner’s syndrome patients free of structural aortic anomalies is comparable with published data with associated lesions. Growth hormone therapy and karyotype had no significant impact; however, longitudinal follow-up is warranted.

To determine longitudinal changes in psychopathology in a cohort of patients 30–43 years after their first cardiac surgery for Congenital Heart Disease (CHD) in childhood, to compare outcomes of the 30- to 43-year follow-up with normative data, and to identify medical predictors for psychopathology.

This study is the third follow-up of this cohort. The first and second follow-ups of this same cohort were conducted in 1990 and 2001, respectively. At all three follow-ups, psychopathology was assessed with standardised, parallel questionnaires. In 2011, subjective health status was assessed by the Short Form-36. Medical predictor variables were derived from medical examinations and medical records.

In this third follow-up, a total of 252 patients participated. Of these, 152 patients participated in all three follow-ups. Over a 30-year period, proportions of patients showing psychopathology decreased significantly.

At the 30- to 43-year follow-up, overall outcomes on psychopathology for the CHD sample were similar or even better compared with normative groups. Subjective health status was also better compared with normative data.

No differences were found between cardiac diagnostic groups. Medical variables that predicted the course of psychopathology over time were as follows: the scar, as judged by the patient, results of the first cardiac surgery, and the number of hospitalisations.

Over a 30-year period, psychopathology decreased in patients with CHD. Levels of psychopathology in these patients, who are now aged between 30 and 54 years, were comparable or even better than normative data.

Little data are published about right ventricular diastolic performance in patients with critical pulmonary valve stenosis after balloon pulmonary valvuloplasty thus far.

A total of 44 patients with isolated critical pulmonary valve stenosis who had undergone balloon valvuloplasty with haemodynamic recordings were enrolled to the study; 33 patients who came for follow-up underwent further imaging by echocardiography after 6 months and their right ventricular functional parameters were compared with 33 control patients of the same age and sex. Out of 33 patients, 21 underwent cardiac MRI with late gadolinium enhancement to assess the presence of right ventricular fibrosis.

The right ventricular systolic pressure (p<0.0001) and right ventricular outflow tract gradient (p<0.0001) decreased acutely (p<0.0001) after balloon valvuloplasty. During follow-up, M-mode left ventricular end diastolic dimension (p<0.001) and end systolic dimension increased (p<0.001), whereas right ventricular end diastolic dimension decreased (p<0.001). Compared with controls, patients (n=33) had significantly reduced tricuspid annular Ea and higher E/Ea (p<0.001). Right ventricular systolic dysfunction was also suggested by reduced tricuspid annular systolic velocity (p<0.001). Late gadolinium enhancement was demonstrated in 13 out of 21 patients with restrictive physiology, which involves the anterior right ventricular outflow tract, anterior wall, and inferior wall. The right ventricular late gadolinium enhancement score correlated positively with age (r=0.7, p<0.001) and right ventricular mass index (r=0.52, p<0.001).

The persistence of right ventricular diastolic dysfunction after relief of chronic pressure overload of critical pulmonary valve stenosis suggests that a factor – other than increase in afterload – is involved in this physiology. Fibrosis is the most likely factor responsible for persistence of restrictive physiology as documented by late gadolinium enhancement.